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    Paget's Disease of the Nipple

    Key Points

    • Paget's disease of the nipple is an uncommon type of cancer that forms in or around the nipple and accounts for about 1% of all breast cancers.
    • Paget's disease of the nipple may be associated with an underlying breast cancer, either intraductal or invasive.
    • Scientists do not know what causes Paget's disease of the nipple, but two major theories have been suggested for how it develops: One theory is that it starts in the skin; the other theory is that it starts in the breast and spreads into the nipple.
    • Symptoms of early-stage disease may include redness or crusting of the nipple skin. Symptoms of more advanced disease often include tingling, itching, increased sensitivity, burning, or pain in the nipple.
    • Paget's disease of the nipple is diagnosed by performing a biopsy if a cytology specimen tests negative.
    • Mastectomy is the usual treatment for Paget's disease of the nipple. Additional treatments, such as radiation or chemotherapy, may be recommended under certain circumstances.

    What Are the Symptoms of Paget’s Disease of the Nipple?

    Symptoms of early Paget’s disease of the nipple include redness and mild scaling and flaking of the nipple skin. Early symptoms may cause only mild irritation and may not be enough to prompt a visit to the doctor. Improvement in the skin can occur spontaneously, but this should not be taken as a sign that the disease has disappeared. More advanced disease may show more serious destruction of the skin. At this stage, the symptoms may include tingling, itching, increased sensitivity, burning, and pain. There may also be a bloody discharge from the nipple, and the nipple can appear flattened against the breast.

    In approximately half of patients with Paget’s disease of the nipple, a lump or mass in the breast can be felt during a physical exam. In most cases, Paget’s disease of the nipple is initially confined to the nipple, later spreading to the areola (the circular area of darker skin the surrounds the nipple) or other regions of the breast. Paget’s disease of the nipple can also be found only on the areola, where it may resemble eczema, a noncancerous itchy red rash. Although rare, Paget’s disease of the nipple can occur in both breasts.

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